Skip to main content
PMC home page
British Heart Journal logoLink to British Heart Journal
. 1985 Nov;54(5):501–508. doi: 10.1136/hrt.54.5.501

Mitral valve prolapse, aortic compliance, and skin collagen in joint hypermobility syndrome.

C E Handler, A Child, N D Light, D E Dorrance
PMCID: PMC481937  PMID: 3902069

Abstract

Mitral valve prolapse was sought clinically and with phonocardiography and M mode and sector echocardiography in 15 women aged 22-57 years with joint hypermobility syndrome. The type III:III + I collagen ratio was measured in skin biopsy specimens and was found to be raised in seven of 10 patients sampled. Thirteen patients had increased aortic wall compliance measured by the continuous wave Doppler ultrasound technique. Ten (67%) patients had mitral valve prolapse shown by auscultatory signs or echocardiography or both--a prevalence at least three times greater than that in the general adult population. It is concluded that if the abnormality of collagen biosynthesis found in skin biopsy samples in these patients is also present in their mitral valve tissue this may predispose them to prolapse of the valve.

Full text

PDF
501

Images in this article

504Image
on p.504
505Image
on p.505

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Alpert M. A., Carney R. J., Flaker G. C., Sanfelippo J. F., Webel R. R., Kelly D. L. Sensitivity and specificity of two-dimensional echocardiographic signs of mitral valve prolapse. Am J Cardiol. 1984 Oct 1;54(7):792–796. doi: 10.1016/s0002-9149(84)80210-6. [DOI] [PubMed] [Google Scholar]
  2. Barlow J. B., Bosman C. K., Pocock W. A., Marchand P. Late systolic murmurs and non-ejection ("mid-late") systolic clicks. An analysis of 90 patients. Br Heart J. 1968 Mar;30(2):203–218. doi: 10.1136/hrt.30.2.203. [DOI] [PMC free article] [PubMed] [Google Scholar]
  3. Barlow J. B., Pocock W. A. The problem of nonejection systolic clicks and associated mitral systolic murmurs: emphasis on the billowing mitral leaflet syndrome. Am Heart J. 1975 Nov;90(5):636–655. doi: 10.1016/0002-8703(75)90229-x. [DOI] [PubMed] [Google Scholar]
  4. Beighton P., Horan F. Orthopaedic aspects of the Ehlers-Danlos syndrome. J Bone Joint Surg Br. 1969 Aug;51(3):444–453. [PubMed] [Google Scholar]
  5. Bisset G. S., 3rd, Schwartz D. C., Meyer R. A., James F. W., Kaplan S. Clinical spectrum and long-term follow-up of isolated mitral valve prolapse in 119 children. Circulation. 1980 Aug;62(2):423–429. doi: 10.1161/01.cir.62.2.423. [DOI] [PubMed] [Google Scholar]
  6. Brown O. R., DeMots H., Kloster F. E., Roberts A., Menashe V. D., Beals R. K. Aortic root dilatation and mitral valve prolapse in Marfan's syndrome: an ECHOCARDIOgraphic study. Circulation. 1975 Oct;52(4):651–657. doi: 10.1161/01.cir.52.4.651. [DOI] [PubMed] [Google Scholar]
  7. Cabeen W. R., Jr, Reza M. J., Kovick R. B., Stern M. S. Mitral valve prolapse and conduction defects in Ehlers-Danlos syndrome. Arch Intern Med. 1977 Sep;137(9):1227–1231. [PubMed] [Google Scholar]
  8. Child J. S., Skorton D. J., Taylor R. D., Krivokapich J., Abbasi A. S., Wong M., Shah P. D. M mode and cross-sectional echocardiographic features of flail posterior mitral leaflets. Am J Cardiol. 1979 Dec;44(7):1383–1390. doi: 10.1016/0002-9149(79)90457-0. [DOI] [PubMed] [Google Scholar]
  9. Cole W. G., Chan D., Hickey A. J., Wilcken D. E. Collagen composition of normal and myxomatous human mitral heart valves. Biochem J. 1984 Apr 15;219(2):451–460. doi: 10.1042/bj2190451. [DOI] [PMC free article] [PubMed] [Google Scholar]
  10. Corrigall D., Bolen J., Hancock E. W., Popp R. L. Mitral valve prolapse and infective endocarditis. Am J Med. 1977 Aug;63(2):215–222. doi: 10.1016/0002-9343(77)90235-2. [DOI] [PubMed] [Google Scholar]
  11. Davies M. J., Moore B. P., Braimbridge M. V. The floppy mitral valve. Study of incidence, pathology, and complications in surgical, necropsy, and forensic material. Br Heart J. 1978 May;40(5):468–481. doi: 10.1136/hrt.40.5.468. [DOI] [PMC free article] [PubMed] [Google Scholar]
  12. DeMaria A. N., King J. F., Bogren H. G., Lies J. E., Mason D. T. The variable spectrum of echocardiographic manifestations of the mitral valve prolapse syndrome. Circulation. 1974 Jul;50(1):33–41. doi: 10.1161/01.cir.50.1.33. [DOI] [PubMed] [Google Scholar]
  13. Devereux R. B., Brown W. T., Kramer-Fox R., Sachs I. Inheritance of mitral valve prolapse: effect of age and sex on gene expression. Ann Intern Med. 1982 Dec;97(6):826–832. doi: 10.7326/0003-4819-97-6-826. [DOI] [PubMed] [Google Scholar]
  14. Goodman D., Kimbiris D., Linhart J. W. Chordae tendineae rupture complicating the systolic click-late systolic murmur syndrome. Am J Cardiol. 1974 May 6;33(5):681–684. doi: 10.1016/0002-9149(74)90263-x. [DOI] [PubMed] [Google Scholar]
  15. Grahame R., Edwards J. C., Pitcher D., Gabell A., Harvey W. A clinical and echocardiographic study of patients with the hypermobility syndrome. Ann Rheum Dis. 1981 Dec;40(6):541–546. doi: 10.1136/ard.40.6.541. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Hammer D., Leier C. V., Baba N., Vasko J. S., Wooley C. F., Pinnell S. R. Altered collagen composition in a prolapsing mitral valve with ruptured chordae tendineae. Am J Med. 1979 Nov;67(5):863–866. doi: 10.1016/0002-9343(79)90746-0. [DOI] [PubMed] [Google Scholar]
  17. Hickey A. J., MacMahon S. W., Wilcken D. E. Mitral valve prolapse and bacterial endocarditis: when is antibiotic prophylaxis necessary? Am Heart J. 1985 Mar;109(3 Pt 1):431–435. doi: 10.1016/0002-8703(85)90543-5. [DOI] [PubMed] [Google Scholar]
  18. Jaffe A. S., Geltman E. M., Rodey G. E., Uitto J. Mitral valve prolapse: a consistent manifestation of type IV Ehlers-Danlos syndrome. The pathogenetic role of the abnormal production of type III collagen. Circulation. 1981 Jul;64(1):121–125. doi: 10.1161/01.cir.64.1.121. [DOI] [PubMed] [Google Scholar]
  19. Lachman A. S., Bramwell-Jones D. M., Lakier J. B., Pocock W. A., Barlow J. B. Infective endocarditis in the billowing mitral leaflet syndrome. Br Heart J. 1975 Mar;37(3):326–330. doi: 10.1136/hrt.37.3.326. [DOI] [PMC free article] [PubMed] [Google Scholar]
  20. Laogun A. A., Gosling R. G. In vivo arterial compliance in man. Clin Phys Physiol Meas. 1982 Aug;3(3):201–212. doi: 10.1088/0143-0815/3/3/004. [DOI] [PubMed] [Google Scholar]
  21. Leatham A., Brigden W. Mild mitral regurgitation and the mitral prolapse fiasco. Am Heart J. 1980 May;99(5):659–664. doi: 10.1016/0002-8703(80)90740-1. [DOI] [PubMed] [Google Scholar]
  22. Light N. D. Estimation of types I and III collagens in whole tissue by quantitation of CNBr peptides on SDS-polyacrylamide gels. Biochim Biophys Acta. 1982 Mar 18;702(1):30–36. doi: 10.1016/0167-4838(82)90024-3. [DOI] [PubMed] [Google Scholar]
  23. Lobstein H. P., Horwitz L. D., Curry G. C., Mullins C. B. Electrocardiographic abnormalities and coronary arteriograms in the mitral click-murmur syndrome. N Engl J Med. 1973 Jul 19;289(3):127–131. doi: 10.1056/NEJM197307192890304. [DOI] [PubMed] [Google Scholar]
  24. Malcolm A. D., Bougher D. R., Kostuk W. J., Ahuja S. P. Clinical features and investigative findings in presence of mitral leaflet prolapse. Study of 85 consecutive patients. Br Heart J. 1976 Mar;38(3):244–256. doi: 10.1136/hrt.38.3.244. [DOI] [PMC free article] [PubMed] [Google Scholar]
  25. Malcolm A. D. Mitral valve prolapse associated with other disorders. Casual coincidence, common link, or fundamental genetic disturbance? Br Heart J. 1985 Apr;53(4):353–362. doi: 10.1136/hrt.53.4.353. [DOI] [PMC free article] [PubMed] [Google Scholar]
  26. Markiewicz W., Stoner J., London E., Hunt S. A., Popp R. L. Mitral valve prolapse in one hundred presumably healthy young females. Circulation. 1976 Mar;53(3):464–473. doi: 10.1161/01.cir.53.3.464. [DOI] [PubMed] [Google Scholar]
  27. Neil-Dwyer G., Child A. H., Dorrance D. E., Pope F. M., Bartlett J. Aortic compliance in patients with ruptured intracranial aneurysms. Lancet. 1983 Apr 23;1(8330):939–940. doi: 10.1016/s0140-6736(83)91376-4. [DOI] [PubMed] [Google Scholar]
  28. Pitcher D., Grahame R. Mitral valve prolapse and joint hypermobility: evidence for a systemic connective tissue abnormality? Ann Rheum Dis. 1982 Aug;41(4):352–354. doi: 10.1136/ard.41.4.352. [DOI] [PMC free article] [PubMed] [Google Scholar]
  29. Pocock W. A., Bosman C. K., Chesler E., Barlow J. B., Edwards J. E. Sudden death in primary mitral valve prolapse. Am Heart J. 1984 Feb;107(2):378–382. doi: 10.1016/0002-8703(84)90389-2. [DOI] [PubMed] [Google Scholar]
  30. Pope F. M., Nicholls A. C., Dorling J., Webb J. Molecular abnormalities of collagen: a review. J R Soc Med. 1983 Dec;76(12):1050–1062. doi: 10.1177/014107688307601215. [DOI] [PMC free article] [PubMed] [Google Scholar]
  31. Pope F. M., Nicholls A. C., Narcisi P., Bartlett J., Neil-Dwyer G., Doshi B. Some patients with cerebral aneurysms are deficient in type III collagen. Lancet. 1981 May 2;1(8227):973–975. doi: 10.1016/s0140-6736(81)91735-9. [DOI] [PubMed] [Google Scholar]
  32. Wood S. J., Thomas J., Braimbridge M. V. Mitral valve disease and open heart surgery in osteogenesis imperfecta tarda. Br Heart J. 1973 Jan;35(1):103–106. doi: 10.1136/hrt.35.1.103. [DOI] [PMC free article] [PubMed] [Google Scholar]

Articles from British Heart Journal are provided here courtesy of BMJ Publishing Group

RESOURCES